Howard J. Zirkin

Overexpression of p53 tumor suppressor gene in pterygia.

Purpose To assess p53 gene expression in pterygia with and without recurrence. The pathogenesis of pterygium has not yet been determined. The most widely recognized etiologic factor is ultraviolet radiation, which leads to degeneration of the conjunctiva. However, pterygium was recently found to have several tumor-like characteristics. The p53 gene is a common marker for neoplasia, and is known to control cell cycle, cell differentiation and apoptosis. In this study we examined the expression of the p53 gene in primary pterygia with and without recurrence, searching for the pathogenesis of this very common lesion and for a prognostic factor for recurrence.Methods Immunohistochemical staining using a monoclonal antibody to human p53 (DO-7) was performed on 13 consecutive patients with primary pterygia, four pterygia without recurrence and nine pterygia which recurred during a 12-month follow-up. As a control we used two specimens of normal conjunctiva.Results Seven of the 13 pterygia specimens (54%) were positive for abnormal p53 expression. There was no difference between the groups with and without recurrence. Two out of four pterygia (50%) without recurrence and five out of nine (55.5%) pterygia with recurrence were positive. No pathological staining was observed in the control specimens.Conclusions In this study, abnormal p53 expression was found in pterygial epithelium, suggesting that pterygium could be a result of uncontrolled cell proliferation, and not as a degenerative lesion. There seems to be no connection between abnormal p53 expression and recurrence.

Necrotizing soft‐tissue infections in the head and neck: A clinicopathological study

The clinicopathological aspects of 15 patients with necrotizing soft‐tissue infections in the head and neck are reviewed. Our relatively large series suggests that the disease occurs more frequently than described in the literature.

Manifestations of three HHV-8-related diseases in an HIV-negative patient: immunoblastic variant multicentric Castleman's disease, primary effusion lymphoma, and Kaposi's sarcoma.

We describe a 73‐year‐old HIV negative patient who presented with symptomatic hypoglycemia. Over the course of several months she was diagnosed with three human herpesvirus‐8 related diseases: multicentric Castlemans disease, primary effusion lymphoma and Kaposis sarcoma. No improvement was observed following cytotoxic therapy and she died 16 months after her initial presentation. The etiology of the hypoglycemia remained obscure over the course of this patients disease. This case is the first report of a patient with three human herpesvirus‐8 related diseases, and the first report of severe hypoglycemia as the presenting symptom of any of these diseases. Am. J. Hematol. 65:310–314, 2000.

Necrotizing soft-tissue infections of dental origin

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Small cell undifferentiated carcinoma of the colon associated with hepatocellular carcinoma in an immunodeficient patient

Small cell undifferentiated carcinoma (oat cell carcinoma) is a malignant epithelial neoplasm with neuroendocrine features. It can appear as a primary tumor in many organs besides the lung, including the colon. We report a case of primary small cell undifferentiated carcinoma of the left colon with omental metastases in a 23-year-old man with a history of X-linked hyper-IgM syndrome. The patient had a simultaneous primary hepatocellular carcinoma. A literature review of this rare colonic malignancy is presented together with a discussion of the possible relationship of this tumor with hepatic malignancy and immunodeficiency.

Toxic effects of subconjunctival 5-fluorouracil and mitomycin C on ciliary body of rats.

Purpose: The effects of subconjunctival injection of mitomycin C and different concentrations of 5-fluorouracil on the epithelium of the ciliary body of twenty Sprague Dawley SD rats was studied.Methods: Twenty rats were divided into four treatment groups.The first three groups received 0.2 ml of 5, 10, and 30 mg of 5-fluorouracil subconjunctivally respectively, and the fourth group 0.2 ml of 0.4 mg/ml mitomycin C subconjunctivally. The right eye received 0.2 ml of the antimetabolite,while the left eye was injected with 0.2 ml of saline subconjunctivally, as a control.The eyes were examined histologically, in a masked fashion, by light and bytransmission electron microscopy. In each treatment group, two eyes were examined after one week, and three eyes were examined one month after the subconjunctival injection. Results: Electron microscopy revealed toxic effects in the epitheliumof the ciliary body of all treatment groups. The 5-fluorouracil group revealed focal mitochondrial edema, enlargement of intercellular spaces, and dilatation of intracellular spaces. The mitomycin C group showed pyknotic nuclei,enlargement of intercellular spaces, and irregular flattened epithelial cells. Theseverity of changes correlated with concentration and length of exposure. No pathology was found by light microscopy in all groups.Conclusions: This study demonstrates that subconjunctival antimetabolites mitomycin C and 5-fluorouracil can penetrate the sclera and exert toxiceffects on the epithelium of the ciliary body, even in low doses. These changeswere only apparent by electron microscopy and were still present one month after theinjection. These findings may contribute to the theory that the application ofantimetabolites during or after surgery has a direct effect on the epithelium of the ciliary body, besides its known effect on the conjunctiva. Further studies are needed to evaluate its effect on intraocular pressure.

Primary bilateral adrenal lymphoma relapsing as a solid cerebral mass after complete clinical remission: A case report

Primary adrenal lymphoma is extremely rare. Only 75 cases have been reported in the medical literature. A case of non-Hodgkin’s lymphoma originating in both adrenal glands is presented. Combination chemotherapy apparently produced complete disappearance of the primary lymphomatous lesions, but subsequently a cerebral relapse was discovered 6 months later, in the form of a solid brain mass. Cranial extension of primary adrenal lymphoma is extremely unusual, and the presentation as a solid mass seems to be unique.

Proliferating trichilemmal tumor of the vulva. Case report and review of the literature.

A case of proliferating trichilemmal tumor, otherwise known as pilar tumor, occurring in the dermis of the left labium majus of a 65-year-old woman, is presented. To our knowledge, this is the second reported case of such a tumor occurring in this location. The origin of this neoplasm, the reasons for its rarity at this site, and the range of its biological behavior are discussed in relation to the clinical manifestations of this case.

Metastatic Angiosarcoma of the Spleen After Accidental Radiation Exposure: A Case Report

Angiosarcoma is a rare malignant tumor arising from endothelial cells of blood vessels or lymphatic channels. Therapeutic irradiation, thoriumdioxide administration, pyothorax, and polyvinyl chloride exposure have been shown to be predisposing factors for developing angiosarcoma. Accidental radiation exposure has not been associated with angiosarcoma. We present an unusual case of angiosarcoma of the spleen, with metastases to bone, liver, breast, and bone marrow, in a woman who lived near the Chernobyl nuclear facility in the former Soviet Union at the time of the reactor accident in 1986. To the best of our knowledge, this is the first report of metastatic angiosarcoma after accidental radiation exposure.

Familial Meconium lleus with Normal Sweat Electrolytes

Meconium ileus (MeI) is the presenting symptom in 10 to 15 percent of infants with cystic fibrosis (CF), but it is a rare phenomenon in patients who do not have this disease. We report four Bedouin siblings who had Mel with no laboratory evidence of CF.